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Background Exacerbations of COPD (ECOPD) have a major impact on patients and healthcare systems across the world. Precise estimates of the global burden of ECOPD on mortality and hospital readmission are needed to inform policy makers and aid preventive strategies to mitigate this burden. The aims of the present study were to explore global in-hospital mortality, post-discharge mortality and hospital readmission rates after ECOPD-related hospitalisation using an individual patient data meta-analysis (IPDMA) design. Methods A systematic review was performed identifying studies that reported in-hospital mortality, postdischarge mortality and hospital readmission rates following ECOPD-related hospitalisation. Data analyses were conducted using a one-stage random-effects meta-analysis model. This study was conducted and reported in accordance with the PRISMA-IPD statement. Results Data of 65 945 individual patients with COPD were analysed. The pooled in-hospital mortality rate was 6.2%, pooled 30-, 90- and 365-day post-discharge mortality rates were 1.8%, 5.5% and 10.9%, respectively, and pooled 30-, 90- and 365-day hospital readmission rates were 7.1%, 12.6% and 32.1%, respectively, with noticeable variability between studies and countries. Strongest predictors of mortality and hospital readmission included noninvasive mechanical ventilation and a history of two or more ECOPD-related hospitalisations < 12 months prior to the index event. Conclusions This IPDMA stresses the poor outcomes and high heterogeneity of ECOPD-related hospitalisation across the world. Whilst global standardisation of the management and follow-up of ECOPD-related hospitalisation should be at the heart of future implementation research, policy makers should focus on reimbursing evidence-based therapies that decrease (recurrent) ECOPD.

Paul Ellis,1,2 Emily Bailey,2 Radmila Choate,3 Kristen E Holm,4,5 Robert A Sandhaus,5,6 Alice M Turner,1,2 Michael Newnham1,2 1Institute of Applied Health Research, University of Birmingham, Birmingham, UK; 2University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK; 3University of Kentucky College of Public Health, Lexington, KY, USA; 4Division of Neurology and Behavioural Health, National Jewish Health, Denver, CO, USA; 5AlphaNet, Kissimmee, FL, USA; 6Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, CO, USACorrespondence: Paul Ellis, Email p.ellis@bham.ac.ukAim: Alpha-1 antitrypsin deficiency is an autosomal co-dominant condition that predisposes individuals to early-onset emphysema. As with COPD, AATD-COPD is associated with pulmonary exacerbations, which impacts on overall mortality and quality of life. Though there is evidence that COPD is associated with a higher prevalence of cardiovascular disease and major adverse cardiovascular events (MACE), it is unclear if this is true for patients with AATD-COPD.Methods: Prevalence of cardiovascular disease was determined in two separate severe AATD cohorts: AlphaNet, USA and the Birmingham AATD registry, UK. All patients had preexisting lung disease. Cardiovascular disease was defined as presence of any of the following: heart failure, ischaemic heart disease, atrial fibrillation, stroke, and myocardial infarction. A Cox proportional hazards model was used to assess the impact of prior cardiovascular disease and frequent exacerbator phenotype on risk of future MACE.Results: Out of 3493 patients with severe AATD, 14.7% had prior cardiovascular disease, including stroke (2.3%), myocardial infarction (2.2%), and heart failure (2.5%). Frequent exacerbators were more likely to have preexisting cardiovascular disease compared with those with one or no exacerbations in the preceding year (63% vs 44.8%, p = 0.001). There was increased risk of future MACE in frequent exacerbato

Background: Although bronchiectasis has been recognised as a feature of some patients with Alpha1-Antitrypsin deficiency the prevalence and characteristics are not widely known. We wished to determine the prevalence of bronchiectasis and patient characteristics. The first cohort of patients recruited to the EARCO (European Alpha1 Research Collaboration) International Registry data base by the end of 2021 was analysed for radiological evidence of both emphysema and bronchiectasis as well as baseline demographic features. Results: Of the first 505 patients with the PiZZ genotype entered into the data base 418 (82.8%) had a reported CT scan. There were 77 (18.4%) with a normal scan and 38 (9.1%) with bronchiectasis alone. These 2 groups were predominantly female never smokers and had lung function in the normal range. The remaining 303 (72.5%) ZZ patients all had emphysema on the scan and 113 (27%) had additional evidence of bronchiectasis. Conclusions: The data indicates the bronchiectasis alone is a feature of 9.1% of patients with the PiZZ genotype of Alpha1-antitrypsin deficiency but although emphysema is the dominant lung pathology bronchiectasis is also present in 27% of emphysema cases and may require a different treatment strategy.

Background: Augmentation therapy (AT) is the only specific treatment licensed for patients with alpha-1 antitrypsin deficiency (AATD) associated lung disease. Since patients with severe AATD may have a very different prognosis and AT requires intravenous infusions for life, the decision to initiate AT may be challenging. Methods: This survey was conducted on 63 experts in AATD from 13 European countries about their opinions and attitudes regarding AT. Participants were asked to rank the importance of 11 identified factors related with the prescription of AT. In addition, each participant was asked to respond to the indication of AT for 30 out of 500 hypothetical cases developed with the combinations of the 11 factors. Each case was evaluated by 3 experts to check the concordance. Results: The variables that scored higher on preferences for initiating AT were AAT genotype (score 8.6 from a Likert scale 0-10 (SD: 1.7)), AATD serum level (8.2 (SD:2.4)) and FEV1 (%) decline (7.9 (SD:2.4)). Among the 500 different cases, there was an agreement in indication of AT among the 3 experts in 291 (58.2%). Regarding the variables associated with AT, it was indicated to 81.9% of Pi*ZZ, 52.4% of Pi*SZ and 9.8% of Pi*MZ (p < 0.0001). For Pi*ZZ patients, multivariate analysis identified younger age, reduced FEV1 (%), higher FEV1 decline and worse emphysema as significantly associated with prescription (AUC = 0.8114); for Pi*SZ variables were younger age, worse FEV1 (%) and worse emphysema (AUC = 0.7414); and for Pi*MZ younger age, worse DLCO (%), higher DLCO decline and dyspnea (AUC = 0.8387). Conclusion: There is a high variability in the criteria for prescription of AT among European experts. Most cases were recommended AT according to guidelines, but a significant number of patients with genotype Pi*SZ and almost 10% Pi*MZ were recommended to initiate AT despite the lack of evidence of efficacy in these genotypes. Keywords: EARCO; Europe; augmentation therapy; survey.

Alpha-1 antitrypsin deficiency (AATD) is characterised by low serum levels of alpha-1 antitrypsin (AAT) and predisposes to the development of pulmonary emphysema and liver disease [1]. This deficiency is considered a rare condition and its prevalence ranges from one in 2400 to one in 6000 individuals in Western European countries [2]. Due to its low prevalence, large observational prospective registries are needed to collect information about the clinical characteristics and prognosis of the individuals affected.

Background The number of people in the UK with two or more conditions continues to grow and their clinical management is complicated by the reliance on guidance focused on a single condition. This leaves individual clinicians responsible for collating disparate information from patient management systems and care recommendations to manually manage the contradictions that exist in the simultaneous treatment of various conditions. Methods/design We have devised a modelling language based on BPMN that allows us to create computer interpretable representations of single condition guidance and incorporate patient data to detect the points of conflict between multiple conditions based on their transformation to logical constraints. This has been used to develop a prototype clinical decision support tool that we can use to highlight the causes of conflict between them in three main areas: medication, lifestyle and well-being, and appointment bookings. Results The prototype tool was used to discern contradictions in the care recommendations of chronic obstructive pulmonary disease and osteoarthritis. These were presented to a panel of clinicians who confirmed that the tool produced clinically relevant alerts that can advise clinicians of the presence of conflicts between guidelines relating to both clashes in medication or lifestyle advice. Conclusions The need for supporting general practitioners in their treatment of patients remains and this proof of concept has demonstrated that by converting this guidance into computer-interpretable pathways we can use constraint solvers to readily identify clinically relevant points of conflict between critical elements of the pathway.

Timm Greulich, 1,* Anna Albert, 1,* Werner Cassel, 2 Tobias Boeselt, 1 Erika Peychev, 1 Andreas Klemmer, 1 Francini Ferreira, 3 Christian Clarenbach, 4 Maria L Torres-Duran, 5, 6 Alice M Turner, 7 Marc Miravitlles 8 1University Medical Centre Giessen and Marburg, Philipps-University, Department of Medicine, Pulmonary and Critical Care Medicine, Member of the German Centre for Lung Research (DZL), Marburg, Germany; 2University Medical Centre Giessen and Marburg, Philipps-University, Department of Medicine, Pulmonary and Critical Care Medicine, Sleep Disorders Centre, Member of the German Centre for Lung Research (DZL), Marburg, Germany; 3Bioclever, Barcelona, Spain; 4Division of Pulmonology, University Hospital Zurich, Zurich, Switzerland; 5Pneumology Department, Hospital Álvaro Cunqueiro, AS Vigo, Spain; 6Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain; 7Respiratory Medicine, University Hospitals Birmingham NHS Foundation Trust, Institute of Applied Health Research, University of Birmingham, Birmingham, UK; 8Pneumology Department, Hospital Universitari Vall d´Hebron/Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron Barcelona Hospital Campus, CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain*These authors contributed equally to this workCorrespondence: Marc MiravitllesPneumology Department, Vall d´Hebron Barcelona Hospital Campus, P. Vall d’Hebron 119-129, Barcelona, 08035, SpainTel/Fax +34 932746083Email marcm@separ.esBackground: Augmentation therapy (AT) is the only specific treatment licensed for patients with alpha-1 antitrypsin deficiency (AATD) associated lung disease. Since patients with severe AATD may have a very different prognosis and AT requires intravenous infusions for life, the decision to initiate AT may be challenging.Methods: This survey was conducted on 63 experts in AATD from 13 European countries about their opinions and attitudes reg